Austrian Syndrome with Quadruple Valve Infective Endocarditis – A Case [email protected] Report and Literature Review of Quadruple Valve Infective Endocarditis SW Zheng1,2, JXJ Soh1,2, H Shafi1 1Division of Infectious Diseases, Changi General Hospital, Singapore; 2Department of Infectious Diseases, Singapore General Hospital, Singapore

BACKGROUND: Figure 1. Imaging A B A B RESULTS: Figure 2. Magnetic Table 1. Cerebrospinal Fluid The triad of , and endocarditis secondary to Streptococcus pictures showing resonance imaging of A total of 19 cases were available, including our patient. The mean age of Biochemistry left lower lobe the brain showing (A) pneumoniae was first described by Heschl in 1862 followed by Osler in 1881. In 1956, Cell count: 225 unit/mm3 presentation was 48.3 years, with a range from 7 to 82 years. There were more pneumonia: (A) cortical infarcts in left RBC: 63 unit/mm3 Austrian described a case series of 8 patients with pneumococcal endocarditis and chest radiograph temporoparietal lobe, men (84.2%) than women (15.8%). Four patients had a history of intravenous Protein: 1.45 g/L rupture of the aortic valve. This triad became known as the Austrian syndrome. (B) computed (B) leptomeningeal drug abuse, another four had underlying congenital heart disease and one had tomography of the Glucose 1.3 mmol/L (capillary blood glucose We present what we believe to be the first reported case of Austrian syndrome with enhancement post 5.7 mmol/L) both. Two patients (10.5%) had two microorganisms isolated. Staphylococcus chest quadruple heart valve involvement, and review the literature detailing cases of gadolinium contrast. aureus and Streptococcus viridans (three cases, 15.8% each) were the most quadruple valve infective endocarditis. commonly implicated microorganism. Heart failure was the commonest A B C D complication, afflicting eight patients (42.1%). Eight patients (42.1%) underwent surgery. Overall, ten patients died, giving a case fatality rate of 52.6%. Cardiac LA surgery was of borderline significance in predicting survival (p = 0.054). LA MV CASE AND METHODS: LA LV AV LA Endocarditis was diagnosed only at post-mortem in two cases (10.5%). • A 54-year-old Malay male teetotaller, presented to our hospital with a two-week Aorta MV LV history of fever, cough and headache. NCC LCC AV RA RCC P PV • He had no past medical history and denied intravenous drug abuse. RVOT LV TV TV V CONCLUSION: RV • Clinical examination revealed an early diastolic cardiac murmur with basal crackles We report the first case of quadruple valve infective endocarditis secondary to in the left lung. , occurring in a patient with Austrian syndrome and Figure 3. Echocardiographic pictures with red arrows showing (A) aortic valve mass 2.0 x 0.4cm (long axis view), (B) mitral valve mass 1.1 x 0.6cm (long axis view), (C) tricuspid valve mass 1.0 • Chest radiograph and computed tomography scan revealed left lower lobe x0.7cm (4-chamber view), (D) pulmonary valve mass 2.4 x 1.0 cm (short axis view). newly diagnosed ventricular septal defect. Risk factors for quadruple valve bronchopneumonia (Figure 1). LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle; MV: mitral valve; AV: aortic valve; TV: tricuspid valve; PV: pulmonary valve; LCC: left coronary cusp; RCC: right coronary cusp; NCC: non-coronary cusp infective endocarditis include congenital heart disease and intravenous drug • He was commenced on intravenous antibiotics. Table 2. Literature Review of Quadruple Valve Infective Endocarditis abuse. Clinicians need to remain vigilant in the presence of these risk factors. • Repeated blood cultures were non-yielding. Ca Age/Sex Underlying Conditions Risk Factors Microbiological Echocardiography Complications Key Antibiotics Surgery Outcome Re se Valvular IVDU Diagnosis Diagnostic Diagnostic f. • A transthoracic echocardiography showed vegetations involving both the mitral and Heart Disease TTE TEE REFERENCES: 1 54 Male VSD Yes No S. pneumoniae No Yes Embolism VAN + CEF No Death NA aortic valves, with aortic regurgitation. 1. Fernandez JP, McKenzie DB, Roberts PR. Four-valve endocarditis caused by group G streptococcus. Heart 2007; 93(9): Multi-organ failure • On the fourth day of hospitalization, he developed right upper limb weakness and 1039 (including heart failure) 2. Piran S, Rampersad P, Kagal D, et al. Extensive fulminant multivalvular infective endocarditis. JACC Cardiovasc Imaging two episodes of generalized tonic-clonic seizures. Valve perforation 2009; 2(6): 787-9. 2 41 Male Hepatitis C No Yes Group G Streptococcus NM NM Shock CP + GEN Yes Survived 1 3. Haghighi ZO, Nikparvar M, Alizadehasl A, et al. A rare case of community-acquired native quadruple-valve endocarditis. J • Magnetic resonance imaging of the brain revealed leptomeningeal enhancement, as 3 39 Nil No Yes Methicillin sensitive S. No Yes Heart failure CLO + GEN Yes Survived 2 Res Med Sci 2014; 19(1): 69-71 well as cortical infarcts in the left temporal and parietal lobes (Figure 2). Female aureus Valve perforation 4. Krake PR, Zaman F, Tandon N. Native Quadruple-Valve Endocarditis Caused by Enterococcus faecalis. Tex Heart Inst J 4 58 Male VSD Yes No NM NM NM Renal failure NM Yes Survived 3 2004; 31: 90-2. • A lumbar puncture performed was biochemically consistent with meningitis (Table 5 64 Male DM, CKD, HTN No No E. faecalis No Yes Renal failure AMP + GEN NM Death 4 5. Seeburger J, Groesdonk H, Borger MA, et al. Quadruple valve replacement for acute endocarditis. J Thorac Cardiovasc 1). Bacterial gram stain and culture were negative. 6 76 Male CKD, COPD, Factor XIII No No E. faecalis No Yes Heart failure AMP + GEN + IMI Yes Survived 5 Surg 2009; 137(6): 1564-5. deficiency G. morbilorum 6. Cao Y, Gu C, Sun G, et al. Quadruple valve replacement with mechanical valves: an 11-year follow-up study. Heart Surg • Urinary Streptococcus pneumoniae antigen was negative but cerebrospinal fluid 7 47 Male VSD Yes No S. pyogenes NM NM NM NM Yes Survived 6+ Forum 2012; 15(3): E145-9. Streptococcus pneumoniae antigen was positive. 8 34 Male Hepatitis B, VSD Yes No Alpha-hemolytic NM NM Multi-organ failure CP + GEN + CLI Yes Death 7 7. Hosseini MT, Quarto C, Bahrami T. Quadruple-Valve Infective Endocarditis and Ventricular Septal Defect. Tex Heart Inst J Streptococcus (including heart failure) 2013; 40(2): 209-10. 8. Natrajsetty HS, Vijayalakshmi IB, Narashimhan C, et al. Purulent pericarditis with quadruple valve endocarditis. Am J Case • A diagnosis of Austrian syndrome with septic brain emboli was made. 9 7 Male Nil No No Methicillin resistant S. Yes Not done Shock VAN then TIG No Survived 8 Rep 2015; 16: 236-9. aureus • Antimicrobial therapy was switched to meningeal doses of vancomycin and 9. Lam D, Rapaport. Four-valve endocarditis with associated right ventricular mural vegetations. Am Heart J 1988; 115(1 Pt 10 46 Male Mental retardation No No S. viridans Yes Not done Heart failure CP No Survived 9 . 1): 189-92. 11 57 Male Nil No No S. gallolyticus No Yes Heart failure CEF + GEN then AMP-SUL + Yes Survived 10 10. Bassetti M, Secchi G, Borziani S, et al. Successful treatment of four-valve native endocarditis caused by Streptococcus • A transesophageal echocardiography (Figure 3) showed quadruple-valve GEN then AMO-CLA bovis. Int J Cardiol 2004; 97: 159-60. 12 82 Male Colon cancer No No Methicillin resistant S. No Yes Embolism NM No Death 11 11. Berstein NE, Freedberg RS, O'Brien FJ, et al. Four-valve endocarditis resulting from Staphylococcus aureus diagnosed by endocarditis, complicated by aortic valvular perforation and regurgitation, as well as aureus biplane transeosophageal echocardiography. Am Heart J 1993; 126: 251-4. the presence of a small ventricular septal defect. 13 31 Male abuse No Yes Corynebacterium Not done Not done Multi-organ failure VAN + GEN + CP + TMP- No Death* 12 12. Farrer W. Four-valve endocarditis caused by Corynebacterium CDC Group I1. South Med J 1987; 80(7): 923-5. (including heart failure) SMX + ERY 13. Hobbs RD, Downing SE, Andriole VT. Am J Med 1982; 72: 164-8. • He was planned for surgery but unfortunately developed multi-organ failure and 14 56 DM, CKD, HTN No No P. aeruginosa Not done Not done Heart failure NM No Death* 13 14. Deonarine B, Lazar J, Gill MV, et al. Quadri-valvular endocarditis caused by Streptococcus mutans. Clin Microbiol Infect passed away. Female S. marcescens Shock 1997; 3: 139-41. 15 48 Male Alcoholic liver No No S. mutans No Yes Heart failure CP + GEN No Death 14 15. Cremieux AC, Witchitz S, Malergue MC, et al. Clinical and echocardiographic observations in pulmonary valve endocarditis. Am J Cardiol 1985; 56:610-3. 16 58 Male No No S. gallolyticus No Not done Heart failure NM Yes Death 15 16. Kramer NE, Gill SS, Patel R, et al. Pulmonary valve vegetations detected with echocardiography. Am J Cardiol 1977; A computerized PubMed search for reports of quadruple valve infective endocarditis 39:1064-7. Cardiac tamponade 17. Henderson RA, Palmer TJ. Echocardiographic diagnosis of infective endocarditis of all four cardiac valves. Int J Cardiol was conducted for the literature review. References from each of the articles 17 24 Male Nil No Yes P. aeruginosa NM NM NM NM No Death* 16 1991; 33:173-5. 18 78 Nil No No S. viridans NM NM Heart failure CP + GEN No Death* 17 obtained by these searches were also reviewed for relevant case reports. Only reports 18. Anwar AM, Nosir YF, Ajam A, et al. Multivalvular infective endocarditis in a tetralogy of fallot. Echocardiography 2008; Female Pericarditis 25(1): 88-90. available in English were included. 19 18 Male Tetralogy of Fallot Yes Yes S. viridans Yes Not done Shock VAN No Death 18 Myocardial infarction VSD: ventricular septal defect; DM: diabetes mellitus; CKD: chronic kidney disease; HTN: hypertension; COPD: chronic obstructive pulmonary disease; IVDU: intravenous drug user; TTE: transthoracic echocardiography; TEE: transesophageal We present our case along with the other case reports in Table 2. echocardiography; NM: not mentioned; VAN: vancomycin; CEF: ceftriaxone; CP: crystalline ; GEN: gentamicin; CLO: cloxacillin; AMP: ampicillin; IMI: imipenem; CLI: clindamycin; TIG: tigecycline; AMP-SUL: ampicillin sulbactam; AMO-CLA: ACKNOWLEDGEMENT: amoxicillin clavulanic acid; TMP-SMX: trimethoprim sulfamethoxazole; ERY: erythromycin We would like to thank Dr Chai SC and Dr Huang WL from the Department of Cardiology, Changi General Hospital, for providing us *Autopsy performed; +Abstract only with the echocardiography pictures.